Isolated Type Immunoglobulin G4 Sclerosing Cholangitis: The Misdiagnosed Cholangiocarcinoma
Journal Title: Journal of Surgery Research and Practice - Year 2020, Vol 1, Issue 1
Abstract
Over the last two decades, Immunoglobulin G4 (IgG4) related disease (IgG4-RD), also known as IgG4 related sclerosing disease or IgG4 associated disease has become a well-known medical problem, with increasing incidence. IgG4-RD is defined as a systemic disease characterized by inflammation and fibrosis of the affected tissues [1,2]. Inflammation usually occurs during the early phase of the disease, whereas fibrosis develops later when diagnosis is delayed. Although it is well known as an immune mediated disease, and there is a growing evidence that the disease is an autoimmune in nature, the precise pathogenesis is yet to be known. It usually results in pseudo tumorous swelling of the affected organs, along with high levels of serum IgG 4 plasma cells [3,4]. The affected tissues usually share similar pathological features, with dense lyphoplasmacytic infiltrate rich in IgG4-positive plasma cells being the hallmark findings [2].
Authors and Affiliations
Safi Khuri
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- EP ID EP698863
- DOI http://dx.doi.org/10.46889/JSRP.2020.1103
- Views 69
- Downloads 0
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